AB0557 THE SPECTRUM OF PEDIATRIC LUPUS: DATA FROM THE KENYA PEDIATRIC RHEUMATOLOGY (KAPRI) REGISTRY
Authors: A. Migowa, J. Orwa, R. Odhiambo
Journal: Annals of the Rheumatic Diseases (2022)

Background Childhood-onset systemic lupus erythematosus (cSLE) is a prototype autoimmune condition characterized by systemic organ involvement, high morbidity and mortality (1-5). A pediatric rheumatology registry is critical in defining the spectrum of pediatric lupus within the region The Kenya Pediatric Rheumatology Registry (KAPRI ) registry offers a unique opportunity to pioneer and spearhead a systematic and organized format in collecting pertinent clinical information that will offer information on current clinical scenarios and offer a platform to conduct other research projects to improve pediatric lupus healthcare in sub-Sahara Africa. Objectives Our objective was to determine the clinic-epidemiological profile of paediatric lupus patients by describing their baseline patient characteristics and clinical features at the Aga Khan University Medical College East Africa who were enrolled into the KAPRI registry from inception in March 2019 to December 2021. Methods All patient records were selected from the KAPRI registry database using the ICD 10 code M32 that denotes Systemic Lupus Erythematosus (SLE) Age, gender, laboratory, clinical features at diagnosis, treatment options offered at time of diagnosis were extracted from the database. Results Among the 207 patients enrolled thus far in the registry, 7 had a diagnosis of cSLE (3.4%). The commonest symptom among the patients were joint pain, fever and rash. Other clinical features and outcomes are highlighted in the Table 1. Table 1. Clinico-epidemiological features of paediatric Lupus patients Patient Gender Age End Organ Involvement Autoantibody profile Treatment Current Clinical Status 1 Female 14 years Arthritis ANA +ve Antiphospholipid antibody +ve (B2 glycoprotein IgA) Declined treatment of Hydroxychloroquine and Methotrexate to seek 2 nd opinion Lost to follow up 2. Female 14 years 9 months Skin ANA +ve Anti ds DNA +ve Hydroxychloroquine Lost to follow up 3. Female 11years Arthritis Interstitial Lung Disease ANA+ve &gt;1:640 (fine granular) Anti Rib +ve Anti P protein +ve Anti SSA +ve Mycophenolate Mofetil Hydroxychloroquine Prednisone 1 dose of Rituximab Stable with minimally active disease 4. Female 15 years 2 months Lupus Gangrene ANA +1:640 (Homogenpous) Anti Sm +ve Anti RNP +ve Anti ds DNA +ve Anti nucleosome+ve Anti ribosome +ve Cyclophosphamide Mycophenolate Mofetil Aspirin Lost to follow up 5. Female 8 years 7 months Neurolupus Lupus Gangrene ANA +VE 1:640(speckled) Anti Sm +ve Anti RNP +ve Anti Ribosome +ve Cyclophosphamide (6 courses) Rituximab (2 courses) Mycophenolate Mofetil Prednisone Hydroxychloroquine Minimally Active Disease 6. Female 6 years 11 months Arthritis Skin rash Class IV Lupus Nephritis ANA+VE (1:320 homogenous) Anti ds DNA +ve Anti nucleosome +ve Mycophenolate Mofetil Hydroxychloroquine Prednisone Minimally Active Disease 7. Female 14 years 6 months Class II Lupus Nephritis ANA VE 1:320 (Homogenous) Mycophenolate Mofetil Hydroxychloroquine Remission Conclusion Over a 2 year period, 57% of our cSLE patients (4 of 7) achieved remission while 43% (3of 7) were lost to follow up. Further studies are required to elucidate predictors of good clinical response and reasons for loss to follow up among our cSLE patients. References [1]Brunner, H., Huggins J and Klein-Gitelman MS. Pediatric SLE—towards a comprehensive management plan Nat. Rev. Rheumatol. 7, 225–233 (2011). [2]Kamphuis, S. and Silverman, E. D. Prevalence and burden of pediatric-onset systemic lupus erythematosus Nat. Rev. Rheumatol. 6, 538–546 (2010). [3]Migowa A, Colmegna I, Hitchon C, Were E, Ng’ang’a E, Ngwiri T, et al. The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya. Pediatric Rheumatology (2017) [4]Trindade, V.C., Carneiro-Sampaio, M., Bonfa, E. et al. An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus. Pediatr Drugs 23, 331–347 (2021). [5]Scott C, Webb K. Pediatric rheumatology in sub-Saharan Africa. Rheumatology (Oxford). 2014;53(8):1357–8. Disclosure of Interests None declared